Epilepsia, 54(Suppl. 1):53–67, 2013 doi: 10.1111/epi.12106
PSYCHIATRIC DISORDERS IN EPILEPSY
Management of psychogenic nonepileptic seizures *W. Curt LaFrance Jr, †Markus Reuber, and ‡Laura H. Goldstein *Neuropsychiatry and Behavioral Neurology Division, Rhode Island Hospital, Brown University, Alpert Medical School, Providence, Rhode Island, U.S.A.; †Academic Neurology Unit, Royal Hallamshire Hospital, University of Sheffield, Sheffield, United Kingdom; and ‡Department of Psychology, Institute of Psychiatry, King’s College London, London, United Kingdom
the following: presentation of the diagnosis, early phase treatment, psychological and pharmacologic interventions, and maintenance management. The aim of this report is to provide greater clarity about the range and current evidence base for treatment for patients with PNES, with the intention of improving the care of patients with PNES and patients who develop PNES as a comorbidity of epilepsy. KEY WORDS: Psychogenic nonepileptic seizures, Epilepsy, Differential diagnosis, Electroencephalography, Video electroencephalography monitoring, Treatment, Pharmacotherapy, Psychotherapy.
SUMMARY The International League Against Epilepsy (ILAE) Neuropsychobiology Commission gave the charge to provide practical guidance for health professionals for the pharmacologic and nonpharmacologic treatment of patients with psychogenic nonepileptic seizures (PNES). Using a consensus review of the literature, an international group of clinician-researchers in epilepsy, neurology, neuropsychology, and neuropsychiatry evaluated key management approaches for PNES. These included
produced a much more detailed report on the investigation and diagnosis of patients with PNES (LaFrance et al., 2013a), the ILAE Neuropsychobiology Commission asked a committee of internationally recognized experts to produce a more detailed report on the treatment of PNES. A summary of the best current practice of the management of PNES compiled by these experts was then reviewed by the members of the ILAE Neuropsychobiology Commission. This article is the outcome of this international collaboration process. Its purpose is to provide specific recommendations for the management for patients with PNES. Management of PNES is divided into four stages; making the diagnosis, presenting the diagnosis, gaining control of the seizures, and management of seizures and life activities.
The International League Against Epilepsy (ILAE) and its national affiliates, U.S. and United Kingdom research funding agencies (National Institutes of Health [NIH] and National Institute of Healthcare Research [NIHR]), and Epilepsy Foundations are increasingly paying attention to seizure disorders other than epilepsy and the comorbidities of epilepsy (Kelley et al., 2009). The ILAE supported an expert consensus report on management of neuropsychiatric conditions in epilepsy (Kerr et al., 2011). Included in the conditions described are nonepileptic seizures (NES) and, more specifically, psychogenic nonepileptic seizures (PNES). Given the absence of a fully powered randomized controlled treatment trial for patients with PNES, national funding agencies are now devoting resources to develop much needed treatments for the condition. The ILAE Neuropsychiatry of Epilepsy consensus document provides an outline of management recommendations for PNES based on the best-known approaches in the field, observational data, and expert recommendations (LaFrance & Devinsky, 2002; Kerr et al., 2011). Having
Making the Diagnosis Best-practice diagnosis should include video-electroencephalography (vEEG) (video telemetry) for each individual with suspected PNES, as well as patients with refractory or pharmacoresistant seizures. Patients with persistent seizures are often treated with antiepileptic drugs (AEDs) for presumed epilepsy in monotherapy or polytherapy. Of the 1% of the U.S. population diagnosed with epilepsy, 5–20% actually have PNES (LaFrance & Benbadis, 2006). Predictors of PNES
Address correspondence to W. Curt LaFrance, Jr, Neuropsychiatry and Behavioral Neurology Division, Departments of Neurology and Psychiatry, Rhode Island Hospital, 593 Eddy St., Providence, RI 02903, U.S.A. E-mail:
[email protected] Wiley Periodicals, Inc. © 2013 International League Against Epilepsy
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54 W. C. LaFrance, Jr et al. include “the rule of 2s,” which includes at least two normal electroencephalography (EEG) studies, with at least two seizures per week, resistance to two antiepileptic drugs (AEDs), yielding an 85% positive predictive value for PNES (Davis, 2004). Although characteristic features of ictal semiology may help distinguish epileptic seizures from PNES (Devinsky et al., 2011), vEEG remains the gold standard for the diagnosis of epilepsy and PNES, and is a test that allows clinicians to establish the diagnosis with a high level of confidence and reliability (Syed et al., 2011). Accurate diagnosis is an essential aid to subsequent management. It is recognized that vEEG monitoring (inpatient or ambulatory EEG with video) is not available throughout the world. Moreover, inpatient vEEG may not be practical in patients with infrequent events, and for patients whose seizures occur only in circumstances unlike those found in a clinical monitoring environment, ambulatory EEG with video may not be accessible. This means that the diagnosis may be arrived at using a combination of history, semiology of the witnessed event, normal routine ictal and interictal EEGs, and a lack of elevated prolactin within 30 min of an apparent generalized tonic–clonic seizure. The relative diagnostic value of these diagnostic techniques and the level of diagnostic certainty that results from their use are described in depth in the recently completed ILAE commissioned paper mentioned above (LaFrance et al., 2013a). The “take home message” is that establishing the diagnosis of PNES, as securely as possible, is the first step in treatment of patients with PNES.
Presenting the Diagnosis In most cases the diagnosis is likely to be communicated by a neurologist. The majority of neurologists accept that the explanation of PNES is part of their role (LaFrance et al., 2008; Mayor et al., 2011), although an early involvement of mental health professionals has also been suggested (Harden & Ferrando, 2001). No research has been undertaken to establish whether it is effective to involve the patients’ family members in the discussion of the diagnosis. However, having family members present during the presentation may facilitate understanding, as described later. Doctors may feel they face a challenge when communicating the diagnosis of PNES. As a group, patients with PNES have experienced more negative life events prior to the development of their seizures than patients who have just developed epilepsy, but they are less likely to accept that these experiences could be relevant to the etiology of their seizure disorder (Binzer et al., 2004). Patients with PNES have an (even more) external health related locus of control than those with epilepsy (Stone et al., 2004). They are more aware of seizure-associated physical (than emotional anxiety) symptoms and may report symptoms Epilepsia, 54(Suppl. 1):53–67, 2013 doi: 10.1111/epi.12106
characteristic of autonomic arousal without recognizing possible subjective emotional experiences associated with these symptoms (Goldstein & Mellers, 2006). In keeping with this, patients with PNES score highly on self-report scales of alexithymia (i.e., indicating difficulty understanding, processing, or describing emotions), although not in a manner that easily distinguishes them from patients with epilepsy (Tojek et al., 2000; Bewley et al., 2005). Unlike patients, neurologists perceive PNES as a largely or entirely “psychological” problem (Whitehead & Reuber, 2012). They consider psychotherapy the treatment of choice for those patients who fail to improve with the communication of the diagnosis (LaFrance et al., 2008, 2012; Mayor et al., 2011). A number of studies have shown how complex the conversations can be, in which neurologists try to “convince” patients with PNES of their own understanding of their disorder. One showed that almost all patients display resistance to the doctor’s attempts to link their apparently physical problem to emotional causes or adverse life events (Monzoni et al., 2011a). Another demonstrated that neurologists seem to anticipate this and treat the communication of the diagnosis of PNES (and that of other “functional” neurological problems) as highly problematic, perhaps provoking patients’ resistance and contributing to patients’ confusion in the process (Monzoni et al., 2011b). Clinical experience suggests that the clinician’s comfort level with explaining a somatoform disorder diagnosis is likely to impact the acceptance by the patient and their family. However, there is increasing evidence that the process of communicating the diagnosis is a very important and potentially effective therapeutic step in the management pathway of patients with PNES. The number of PNES was reduced in the 24 h after the diagnosis was explained in one study (Farias et al., 2003). However, in contrast to the finding of immediate PNES reduction, the 1-year followup showed persistence of seizures in 87% of patients (Wilder et al., 2004). Several retrospective studies suggest that about one third of patients will report that PNES have stopped when asked 3–6 months after diagnosis with no further intervention (Aboukasm et al., 1998; Kanner et al., 1999; Arain et al., 2007). A prospective singlecenter audit showed that nearly one half of patients with recent-onset seizures were PNES-free 6 months after the diagnosis. Most patients who became PNES-free stopped having seizures immediately after the explanation of the condition (McKenzie et al., 2010; Duncan et al., 2011). Likewise, one prospective multicenter study confirmed that PNES can cease with the explanation of the diagnosis alone—although in this study only 16% of patients were PNES free at 6 months of follow-up (Mayor et al., 2010). So far it is uncertain which patients are particularly likely to stop having PNES with the communication of the
55 Management of Psychogenic NES diagnosis alone. However, predictors of persistence of seizures include depression, personality disorder, and abuse history (Kanner et al., 1999). Proposed predictors of PNES cessation include recent onset, the absence of comorbid anxiety, depression, personality disorder or abuse history, and continued employment at the time of diagnosis/lack of reliance on state financial benefits. It may be that the level of diagnostic certainty at the time of the explanation of the diagnosis is relevant. Whereas the diagnosis had been proven by vEEG in almost all patients in the study by Duncan et al. (2011), (McKenzie et al., 2010), about one half of the patients in the study by Mayor et al. (2010) had diagnoses based on clinical features alone. The communication of the diagnosis seems to have an even more impressive immediate effect on healthcare utilization than on seizure control. Several studies have demonstrated reductions in health care expenditure overall or in the use of emergency services more specifically (Martin et al., 1998; McKenzie et al., 2010; Razvi et al., 2012). Of interest, reductions in emergency service use were even seen in those patients who continued to experience PNES (McKenzie et al., 2010). It is important to note that even patients whose PNES stop (at least temporarily) after the explanation of the diagnosis may still need further active psychological or psychiatric treatment. Across the whole PNES patient group, the impact of the explanation of the diagnosis on measures of psychological distress, functioning, or healthrelated quality of life is not impressive. The biggest prospective study of this issue showed no significant change in self-report measures after 6 months, even when PNES had improved or stopped (Mayor et al., 2012b). However, the risk of developing other somatoform problems when PNES have ceased may be smaller than often thought (at least in the short term) (McKenzie et al., 2011). Several studies have demonstrated that the explanation of the diagnosis of PNES may also have adverse consequences. Many patients’ seizures do not experience a sustained improvement of their PNES with the relaying of the diagnosis. They may even show an increase in PNES frequency or experience an exacerbation of other mental health symptoms following delivery of the diagnosis. The likelihood of engaging patients in further treatment (such as psychological therapy) may be reduced if the explanation of the diagnosis received leaves the patient angry or confused (Carton et al., 2003; Thompson et al., 2009). To maximize the possibility of a positive outcome and to reduce the risk of an ineffective discussion, four reasonably detailed communication strategies have been published (see Table 1) (Shen et al., 1990; Mellers, 2005; Duncan, 2010; Hall-Patch et al., 2010). If PNES had been captured by vEEG, all proposed strategies would begin with a search for confirmation that the recorded events were typical of the patient’s habitual events. The strategy
proposed by Shen also involves clinicians showing patients and caregivers a video-recording of the PNES prior to delivering the explanation of the diagnosis. Not surprisingly there is considerable overlap between the strategies. One difference between the approaches is the discussion of etiology. The Shen model, for example, takes a “noncommittal” approach (stating “We may never know what these seizures are…”). A fifth approach to the discussion of the etiology communicates the understanding that PNES have two main causes, developmental emotional privation and acute or remote trauma (Kalogjera-Sackellares, 1996). Unfortunately, there are no comparative studies to guide practitioners in the route they should follow in those areas in which the strategies diverge. Only one of these strategies (consisting of a crib sheet for neurologists and a booklet for patients) has been subjected to a prospective study confirming that patients found the approach acceptable and that the strategy was effective at communicating the possibility of a “psychological” etiology of PNES (Hall-Patch et al., 2010). One in six patients who received the diagnosis in this way reported being PNES free 6 months later (Mayor et al., 2012b). What the condition is called is a key feature of several of the communication approaches summarized in Table 1. The most appropriate name for PNES has sparked particular debate. It is clear that some possible labels (such as “hysterical seizures” and “pseudoseizures”) can offend patients (Stone et al., 2003). It is debatable whether the terms “attack” (differentiating PNES from epileptic “seizures” but potentially associating them with a traumatic attack sustained by patients) or “seizure” (communicating that the doctor is taking the problem seriously but associated with a potential risk of confusion with epileptic seizures) is most suitable (LaFrance, 2010). One small linguistic study of 13 patients with PNES suggested that they treated both terms as problematic (Plug et al., 2009). More important than the preferred label (or whether a label is used at all) is likely to be how empathetically the diagnosis is presented, and whether the doctor communicates that s/he has understood the patient’s account of the problem. It is likely to be helpful if the person communicating the diagnosis has a thorough understanding of epilepsy and PNES and is able to communicate the diagnosis with conviction. Given that it is one of the aims of the discussion to modify patients’ thoughts about their condition, and considering that patients may share unhelpful illness perceptions with family members or relevant others, encouraging patients to bring someone along when the diagnosis is discussed with them is preferred. Ideally, these significant others can help take in what the doctor has to say and help to reinforce the information after the encounter. It is also essential that the diagnosis is communicated clearly to other doctors involved in the patient’s care (i.e., copy the Epilepsia, 54(Suppl. 1):53–67, 2013 doi: 10.1111/epi.12106
56 W. C. LaFrance, Jr et al. Table 1. Strategies used for the communication of the diagnosis of psychogenic nonepileptic seizures Shen et al. (1990) Good news—the seizures are not caused by epilepsy, explain vEEG findings. Bad news—we do not know the precise cause of the seizures but: They are nonepileptic, antiepileptic drugs do not work. Antiepileptic drugs may cause serious side effects. “We may never know what these seizures are but can work together on the problems.” In most cases seizures are eventually related to upsetting emotions of which patients are unaware. This may be best addressed by psychiatrist, psychologist or counselor. You are not crazy, the seizures occur at a subconscious level. Counseling may not control seizures immediately, but seizures can improve as treatment progresses. Neurologic follow-up will continue. A history of sexual abuse is discovered in many cases. The seizures may stop spontaneously. Although they are subconscious, a conscious effort can sometimes stop them. More seizures may occur before complete control is achieved.
Mellers (2005)
Duncan (2010)
Hall-Patch et al. (2010)
Cover reasons for concluding they do not have epilepsy. Relay what they do have (explain “switching off”; describe dissociation). Emphasize they are not suspected of “putting on” the events. They are not “mad”; the problem is common, and seizures are disabling. Events are stress related, but stresses may be difficult to identify. Triggering “stresses” may not be immediately apparent. Relevance of etiologic factors in their case. Maintaining factors. Worry about seizures may make them worse/more frequent. Avoidant behavior may make seizures worse. May improve after correct diagnosis. Caution that AED withdrawal should be gradual. Describe psychological treatment. Include patients’ caregivers when delivering this explanation.
Explain how vEEG works and how it has helped with the diagnosis. Seizures are emotional/ psychological, due to past/ present issues, not a medical condition. List possible predisposing factors as “specimen causes” not directly linked to the patient. Seizures are not under conscious control but patients can learn to control them with help from a therapist. Patients may have anxiety or low mood but are otherwise not mentally ill or “mad.” Drug treatment does not work, psychological treatment can work, no other treatment is available. Describe psychological intervention. Ask whether patients want psychological intervention.
Genuine symptoms. Real events—can be frightening or disabling. Label. Give a name for the condition. Give alternative names they may hear. Reassure that this is a common and recognized condition. Cause and maintaining factors: Not epilepsy, predisposing factors difficult to identify, precipitating factors can be related to stress/emotions, perpetuating factors (vicious cycle: worry or stress/events get worse/more worry). Provide a model for the events—e.g., brain becomes overloaded and shuts down. Treatment. Antiepileptic drugs are not effective. Evidence that psychological treatment is effective. Talk about referral to a treatment specialist. Expectations. Can resolve. Can expect improvement.
medical record of the interview/examination to the other treatment providers), so that the considerable risk of diagnostic confusion and re-prescription of AEDs is minimized; one study showed that 4 years after diagnosis of PNES and withdrawal of AEDs, 40% of patients were taking AEDs again (Reuber et al., 2003b). Although the short-term outcome (at least in terms of self-reported seizure control) of “minimal” therapeutic interventions such as the explanation of the diagnosis (or a brief psychoeducation approach) is relatively well documented now, the encouraging short-term outcomes are not matched by those seen over the longer term (Reuber et al., 2003b; Wilder et al., 2004). Some early relapses after initial seizure cessation have been described even in the short term (Duncan et al., 2011). It is likely that some patients can learn to control their PNES in the long term with minimal interventions, whereas most need more intensive treatment. Although there are no sufficiently sized comparative studies, a short (50 patients) have suggested that the treatment has clinically meaningful effects on seizure frequency and severity, psychological distress, quality of life, and functioning in the short term (Reuber et al., 2007a); that the effect on seizures is maintained in the long term (Mayor et al., 2010), and that the treatment is cost-effective (Reuber et al., 2007a; Mayor et al., 2010). The therapeutic approach is an adaptation of the model of brief PIT developed by Hobson (1985). The original model was found to have equivalent effects to cognitive-behavioral therapy for the treatment of depression (Shapiro & Firth, 1987), and an adapted model for functional somatic disorders, on which this therapy is based, was shown to be helpful and costeffective in the treatment of functional bowel disorders (Guthrie et al., 1991; Creed et al., 2003). The therapy uses an accessible, empathic approach, inviting correction and collaboration with the patient. Key features include (1) the assumption that the patient’s problems arise from or are exacerbated by disturbances of significant personal relationships, with dysfunctional interpersonal patterns usually originating earlier in their lives, and the explicit linking of this to the patient’s symptoms; and (2) a tentative, encouraging, supportive approach from the therapist, using the terms “I” and “we” to emphasize the collaborative nature of the work. Understanding hypotheses are used to develop awareness of the patient’s current feelings (e.g., “I guess you might be feeling quite angry when you remember that”). “Linking
61 Management of Psychogenic NES hypotheses” are introduced to make connections between current feelings and other feelings both inside and outside therapy (e.g., “You say you’re feeling small and frightened now – I wonder if that’s a bit like how you felt as a child when your parents used to fight?”). “Explanatory hypotheses” look for possible underlying reasons for a patient’s behavior, particularly a repeated pattern of behavior (e.g., “When you try so hard not to get upset here with me, maybe it’s because your dad used to beat you more if you cried, so you came to feel that showing your feelings was bad and dangerous. Maybe it even feels as if it might make me angry”). The key mechanisms for therapeutic progress are seen as the identification and change of unhelpful patterns of interpersonal relationships, and the more effective processing of emotions, particularly in relation to painful memories or areas of patients’ lives that may not have been dealt with previously. Because of the florid, easily triggered symptomatology and level of psychological traumatization of many patients with PNES, the augmented brief PIT for PNES combines this approach with concepts and techniques from a model of somatic trauma therapy, which includes techniques to control autonomic arousal, to track somatic symptoms and link them with emotional triggers, and to process traumatic memories without retraumatizing potentially fragile patients (Rothschild, 2000). In practical terms this approach involves an initial extended session in which the patient is engaged and in which a diagnostic formulation is developed. Up to 19 subsequent sessions then use the approach described above to change the patients’ illness perceptions, achieve symptom control, improve emotional processing, increase independence, encourage self-care, and process trauma. The support of family, caregivers, and other health care professionals is enlisted if possible (Howlett & Reuber, 2009). Other interventions A number of other interventions have been studied either only in single case studies, small group studies, or in studies where the main patient group had other (especially motor) conversion disorders. Therefore, for example, although hypnosis has been tested as a diagnostic tool for PNES, with varying levels of sensitivity and specificity when PNES patients are compared to people with epilepsy (Kuyk et al., 1995, 1999; Barry et al., 2000; Khan et al., 2009), and studies have also shown that patients with PNES obtain higher scores than patients with epilepsy on measures of hypnotizability (Kuyk et al., 1999; Barry et al., 2000; Khan et al., 2009), thereby raising expectations of the potential utility of hypnosis as a therapeutic tool for PNES, little explicit use has been made of hypnosis in the treatment of PNES, and there is no robust evidence to recommend its use as a primary intervention for PNES, even when administered by an experienced hypno-
therapist. However, a number of single case reports of its use as an adjunctive therapy can be found (e.g., Stonnington et al., 2006). Accounts of its use in motor conversion disorder, where hypnosis has been used directly and indirectly to influence the relevant symptoms or explore events likely to have triggered the symptoms (Moene & Hoogduin, 1999) have indicated that its use may not always be without problems, and other psychopathology may give rise to unexpected responses or the need to modify the hypnotic induction technique (Moene & Hoogduin, 1999). In RCTs of a hypnosis-based treatment versus waiting list for motor conversion patients (of whom only a minority had seizures as their main symptom), no data were presented specifically in terms of outcome for PNES occurrence (Moene et al., 2002, 2003). Although eye movement desensitization and reprocessing (EMDR) has a strong evidence base for the treatment of posttraumatic stress disorder (e.g., Hogberg et al., 2008), there is no evidence for its use as a primary intervention in patients with PNES beyond the case series level (Chemali & Meadows, 2004; Kelley & Benbadis, 2007), or incorporated within a more complex intervention (Howlett & Reuber, 2009). Similarly although EEG biofeedback has been evaluated as a treatment for epilepsy, the use of sensorimotor theta biofeedback has been evaluated only at the level of single cases for PNES (Swingle, 1998), and then as an adjunct to psychotherapy rather than as a treatment in its own right. In one small study where there was (rather poorly reported) random allocation of patients to treatment groups (behavior therapy vs. pharmacotherapy and outpatient psychiatric review), behavior therapy (the use of positive reinforcement for seizure-free behavior and punishment—to reduce inappropriate behavior—as well as avoiding the use of negative reinforcement) was reported to lead to a reduction in PNES frequency, anxiety, and depression (Aamir et al., 2011). In an earlier study (Ataoglu et al., 1998, 2003), a paradoxical intention approach (where, for example, patients were instructed to imagine situations where they were likely to have their seizures or to provoke seizures) suggested a greater improvement in terms of seizure reduction and improvement in anxiety scores than in patients treated with diazepam. However, this therapeutic approach has not generated sufficient interest to provoke replication in more robust studies. Group therapies Group therapies have focused on psychoeducational approaches to intervention, using a multisession group approach (Myers & Zaroff, 2004; Zaroff et al., 2004) with mixed results on seizure occurrence but improvement in psychological well-being (Zaroff et al., 2004). Group therapy with a psychodynamic focus, which conceptualized the seizures as an expression of unconscious/ Epilepsia, 54(Suppl. 1):53–67, 2013 doi: 10.1111/epi.12106
62 W. C. LaFrance, Jr et al. hidden emotions, has been undertaken (Barry et al., 2008), but with only small numbers of patients. Pilot data from seven female patients completing at least 75% of 32 weekly 90-min–long sessions suggest, based on measures of depression, global symptom severity, and PNES frequency, that there may be some benefit in using this approach as an adjunct to individual psychotherapy. However, the numerous methodologic limitations of this pilot study would necessitate further careful study of this approach. 2. Family therapy may be indicated if family system dysfunction is present. Families of patients with PNES have higher levels of family dysfunction than patients with epilepsy (Krawetz et al., 2001). Patients with PNES see their families as having less commitment and support for each other compared to patients with epilepsy (Moore et al., 1994). Family dysfunction is a contributor to symptoms of depression and to poorer quality of life in PNES (LaFrance et al., 2011). Given these findings, aspects of family dysfunction may be a treatment target in PNES. A well-studied model used for family therapy is the McMaster’s approach (Ryan et al., 2005). The problem-centered, systems-based model addresses affective responsiveness, affective involvement, problem solving, roles, behavior control, communication, and transactional patterns in families (Miller et al., 1985). The systems approach addresses the isolating and restricting tendencies of the patient with PNES in the context of his or her social environment, which may influence integration into the community (LaFrance & Devinsky, 2004). The model has been used successfully in cases of PNES (Archambault & Ryan, 2010). Controlled trials of family therapy for patients with PNES are needed to assess efficacy. (d) The pharmacologic treatment of patients should begin with early tapering and discontinuation of AEDs, which are an ineffective treatment for people with lone PNES, unless a specific AED has a documented beneficial psychopharmacologic effect in an individual (e.g., use for bipolar disorder or as a treatment for migraine). It has been shown that the withdrawal of inappropriately prescribed AEDs is safe for people without comorbid epilepsy and that immediate as opposed to delayed AED withdrawal may have greater beneficial effects on a range of clinical outcomes (Oto et al., 2005, 2010) including seizures and health service use. The importance of early AED withdrawal lies partly in communicating to the patient that they do not have epilepsy and thus that such medication is unwarranted. In view of the potential teratogenic effects of some AEDs, this assumes additional importance for women of child-bearing age, who make up the majority of people with PNES. Epilepsia, 54(Suppl. 1):53–67, 2013 doi: 10.1111/epi.12106
(e) In people with mixed epileptic seizures (ES) and PNES, reduce high doses of AEDs or polytherapy if possible. More rigorous studies show that approximately 10% of patients with PNES have epilepsy (Benbadis et al., 2001). In cases of mixed ES/PNES, identifying the different ictal semiologies of the ES and PNES is essential for directing treatment to the different etiologies. For the epilepsy, reduction of the AED dose to the minimum required to achieve optimal freedom from epileptic seizures was shown to be effective (Blumer & Adamolekun, 2006), given that AEDs can exacerbate PNES (Niedermeyer et al., 1970). AED toxicity was found to result in an increased seizure frequency in patients with PNES (Krumholz & Niedermeyer, 1983). Treating the ES with AEDs and the PNES with psychotherapy allows for targeted interventions for the different etiologies. Good communication between the neurologist/epileptologist and the health professional providing psychological treatment is needed to keep the patients with mixed ES/PNES out of the emergency department with recurrences of PNES. (f) Use psychopharmacologic agents to treat comorbid mood, anxiety, or psychotic disorders, and possibly to treat somatoform symptoms directly. Psychopharmacologic interventions for PNES have been used to treat the somatoform disorder directly and to treat the common comorbidities (LaFrance & Blumer, 2010). Medication treatment approaches historically have been prophylactic or symptomatic. As of yet, no acute pharmacologic treatment for PNES has been developed, except for stopping convulsions with excessive sedation and paralytic agents, used in psychogenic nonepileptic status (Walker et al., 1996). While paralysis, intubation, and coma-pharmacoinduction are indicated in epilepsy status, this is not the appropriate treatment algorithm for the patient with PNES-status, who is not at risk of brain damage from the seizure. Consulting treatment providers familiar with PNES during the acute presentation may mitigate iatrogenesis. Open-label trials of antidepressants in patients with conversion disorders have shown some response (O’Malley et al., 1999; Varia et al., 2000; Voon & Lang, 2005). Phase III controlled studies of the benefit of psychotropics in patients with PNES, however, have not been conducted, and apart from anecdotal reports, their effect is unknown (LaFrance & Barry, 2005). The use of pharmacologic treatments for PNES with intravenous barbiturates, tricyclic antidepressants, selective serotonin reuptake inhibitors (SSRIs), mixed mechanism antidepressants, dopamine receptor antagonists, beta-blockers, analgesics, or benzodiazepines has largely been reported anecdotally in case reports, journal review articles, or book chapters, with only three prospective open-label trials (Ataoglu et al., 1998; LaFrance et al., 2007b; LaFrance & Blumer,
63 Management of Psychogenic NES 2010; Pintor et al., 2010). Only one double-blind placebocontrolled pilot RCT for PNES has been published (LaFrance et al., 2010). Thirty-eight patients enrolled, and 26 (68%) completed the trial. Thirty-three subjects with nonzero baseline seizure rates were included in an intentionto-treat analysis of the primary outcome. Patients assigned to the sertraline arm experienced a 45% reduction in seizure rates from baseline to final visit (p = 0.03) versus an 8% increase in placebo (p = 0.78). The pilot study was not powered for efficacy but showed feasibility for a pharmacologic RCT. Data from this RCT and other openlabel trials indicated that medications may help to reduce symptoms, but would likely require adjunctive psychotherapy to eliminate seizures.
Treatment Maintenance Good communication between treatment providers and a coordinated care approach should prevent further unnecessary interventions, investigations, or treatments. The longer-term studies currently available suggest that many patients with PNES will continue to experience seizures despite neurologic and psychotherapeutic care (Reuber et al., 2003b). Even patients who become free of seizures may remain disabled (Reuber et al., 2005b). Given the association of PNES with serious and pervasive conditions such as borderline personality disorder, PTSD and somatization disorder (at least in important subgroups of patients) (Reuber et al., 2003a; Lacey et al., 2007), it is not surprising that many patients remain symptomatic and disabled. Some patients with chronic seizure disorders (and their families) may have become dependent on health-related benefits associated with PNES. While experts think that only a small minority of individuals (
